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Published on: June 10, 2017
Syncytial variant of nodular sclerosing Hodgkin lymphoma in children: A prognostic factor?
Noa Granot1, Ayelet Ben-Barak2, Yael Fisher3
1a The Bruce Rappaport Faculty of Medicine , Technion-Israel Institute of Technology , Haifa , Israel.
Insights
Syncytial variant (SV) Hodgkin lymphoma (HL) in children, though rare, indicates a poor prognosis. This aggressive form of pediatric HL requires intensive treatment, and its histology may predict outcomes.
Area of Science:
- Pediatric Oncology
- Hematology
- Pathology
Background:
- Nodular sclerosing Hodgkin lymphoma (HL) typically has an excellent prognosis in children.
- The syncytial variant (SV) of HL in adults is associated with a worse outcome.
- Limited data exists on the syncytial variant of HL in pediatric populations.
Purpose of the Study:
- To report the clinical features and disease course of three children with refractory syncytial variant Hodgkin lymphoma.
- To evaluate the syncytial variant as a potential prognostic factor in pediatric HL.
Main Methods:
- Retrospective multi-institutional study.
- Analysis of 51 children diagnosed with refractory or recurrent HL between 1997 and 2014 in Israel.
- Detailed review of three pediatric cases with syncytial variant HL histology.
Main Results:
- All three pediatric patients with SV-HL experienced multiple, rapid recurrences.
- Intensive treatment regimens were utilized, including multiple chemotherapy combinations, autologous and allogeneic bone marrow transplantation, and immunotherapy.
- Outcomes varied: one patient died of disease, one achieved complete response but developed a secondary malignancy, and one remains alive with no evidence of disease.
Conclusions:
- Syncytial variant histology may represent a poor prognostic factor in children diagnosed with Hodgkin lymphoma.
- Further research is warranted to understand the specific behavior and optimal management of SV-HL in pediatric patients.
Abstract:
Nodular sclerosing Hodgkin lymphoma (HL) has an excellent prognosis in children. The syncytial variant (SV) of HL in adults represents a clinic pathologic entity with a worse outcome. We report the clinical features and the course of the disease of three children with refractory HL. The three patients with SV were analyzed in a retrospective multi-institutional study conducted in Israel in 51 children diagnosed with refractory or recurrent HL between 1997 and 2014. All the three children developed multiple recurrences soon after diagnosis. All three received at least three different chemotherapy combinations with autologous bone marrow transplantation for two patients, allogenic bone marrow transplantation in one, and immunotherapy in one. One patient died of disease, one is in complete response of the disease but developed a second metastatic malignancy, and one is alive without disease. This retrospective study shows that SV histology may be a prognostic factor for poor outcome in children diagnosed with HL.
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